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Rehabilitation in Transverse Myelitis

Article  in  CONTINUUM Lifelong Learning in Neurology · August 2011


DOI: 10.1212/01.CON.0000403797.10612.d3 · Source: PubMed

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Cristina L Sadowsky Daniel Becker


Kennedy Krieger Institute Johns Hopkins Medicine
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Glendaliz Bosques Janet M Dean


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Review Article

Rehabilitation in
Address correspondence to
Dr Cristina L. Sadowsky,
Kennedy Krieger Institute,
707 North Broadway, Suite
518, Baltimore, MD 21205,
sadowsky@kennedykrieger.org.
Relationship Disclosure:
Transverse Myelitis
Drs Sadowsky and Cristina L. Sadowsky, MD; Daniel Becker, MD;
Bosques report no
disclosures. Dr Becker has Glendaliz Bosques, MD, FAAPMR; Janet M. Dean, MS, RN, CPNP, CRRN;
received compensation for John W. McDonald III, MD, PhD; Albert Recio, MD, RPT, PTRP;
expert witness testimony
and plans to continue to Elliot M. Frohman, MD, PhD, FAAN
be available for litigation
consultation. Dr Becker
has also received a grant
from the Bryon Riesch
ABSTRACT
Paralysis Foundation for a The consequences of neurologic injuries related to transverse myelitis (TM) are long-
clinical trial. Ms Dean has lasting and require rehabilitative interventions in about two-thirds of cases. Because
received an honorarium
for a speaking numerous neural repair mechanisms are dependent on maintenance of an optimal
engagement at the Rare amount of activity both above and below the injury level, rehabilitation and exercise are
Neuroimmunologic useful not only for compensatory functional purposes but also as tools in neural system
Disorders CME
Conference. Dr McDonald restoration. The application of established neurophysiologic principles to post-TM reha-
may receive a potential bilitation has substantial impact on optimizing residual functional capabilities while
royalty related to a patent facilitating the processes of central plasticity and reorganization of sensory and motor
licensed through
Washington University programming. The process of neurorehabilitation thereby serves both to treat the pa-
School of Medicine from tient with TM and to help physicians interrogate and dissect the mechanisms involved
Restorative Therapies, Inc. in spinal cord injury, neuroprotection, and, ultimately, recovery. Post-TM rehabilitation
Dr Recio has received
grants from the US is lifelong and should be integrated into daily living in a home setting as part of the
Department of Defense. global management of paralysis, a chronic condition with significant comorbidities.
Dr Frohman has received
personal compensation for
speaking engagements or Continuum Lifelong Learning Neurol 2011;17(4):816–830.
consulting activities from
Abbott Laboratories,
Acorda Therapeutics,
Bayer, Biogen Idec, tage for a given individual (resulting
Novartis, and Teva INTRODUCTION
Neuroscience. from an impairment or disability) that
In medicine, rehabilitation is defined as
Unlabeled Use of limits or prevents the fulfillment of a
Products/Investigational the process of assisting someone to im-
role that is normal for that individual,
Use Disclosure: prove or recover lost function after an
Dr Sadowsky discusses depending on age, sex, and social and
event, illness, or injury that has caused
the use of activity-based cultural factors.1
restorative therapy and functional limitations. The goal of reha-
functional electrical With such a broad spectrum of ap-
bilitation is usually to restore the lost
stimulation for neural plication, rehabilitation of a neurologic
restoration. Drs Becker, function or return it to a level as close as
Bosques, McDonald, injury and its consequences should
possible to the one exhibited prior to
Recio, and Frohman and
the injury. Thus, the definition of reha- focus on restoration of both physiologic
Ms Dean report no
disclosures. bilitation is closely related to function. (impairment) and day-to-day (disability)
Copyright*2011, Function is described according to function. While ability-restoring reha-
American Academy of
Neurology. All rights the model in which it is studied. Im- bilitation (or habilitation when the
reserved. pairment is any loss or abnormality of process addresses function potentially
psychological, physiologic, or anatomi- lost from a disease that strikes a child
cal structure or function. Disability is a during development, before he or she
restriction or lack of ability to perform had a chance to acquire such function)
an activity in the manner or within the has long been recognized by the
range considered normal for a human current medical model, impairment-
being as a result of an impairment. A reversing (re)habilitation is not a con-
handicap is described as a disadvan- cept widely accepted yet. There are

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KEY POINTS
several reasons for this, including the ambulation. A greater number of white h Rehabilitation of a
inability to perfectly repair damage blood cells in CSF predicts worse func- neurologic injury and its
and the lack of correlation between tional mobility, as does age younger consequences should
anatomical and physiologic integrity. than 3 at onset of disease.4 A high CSF focus on restoration of
Additionally, the damaged substrate interleukin (IL)-6 level in TM predicts both physiologic
may be unknown or not ‘‘visible’’ using acute and long-term disability.5 Virtually (impairment) and
current medical assessment tools, mak- all patients with acute TM require day-to-day (disability)
ing it unable to be repaired. In trans- acute rehabilitation. Based on the data function.
verse myelitis (TM), all of the above referenced above, it can be concluded h Approximately one-third
factors play a role, but rehabilitation ap- that approximately 66% of all patients of patients with
proaches can be expanded to include with TM will also require long-term transverse myelitis
concepts of impairment and ability re- rehabilitation. recover with little to no
pair and restoration. sequelae, one-third are
TM is defined as a monofocal left with a moderate
TRADITIONAL REHABILITATION degree of permanent
inflammatory process of the spinal
IN TRANSVERSE MYELITIS disability, and one-third
cord. It can exist as part of a multifocal
have severe disabilities.
CNS disease (eg, multiple sclerosis, Functional goals for ‘‘traditional’’ TM re-
neuromyelitis optica), a CNS infection habilitation (the rehabilitation con- h Functional goals for
‘‘traditional’’ transverse
(eg, syphilis, Lyme disease, HIV, human cerned with restoring abilities) include
myelitis rehabilitation
T-cell lymphotropic virus type I, Myco- maximizing physical independence, ca-
include maximizing
plasma, herpesviruses), a multisystem pabilities, and potential. An interdisci- physical independence,
disease (eg, systemic lupus erythemato- plinary team approach is used, and the capabilities, and potential.
sus, sarcoidosis, Sjögren syndrome, team leadership varies according to the This type of rehabilitation
Beh0et disease), or as an isolated idi- goal or task (ie, if the goal is gait train- uses an interdisciplinary
opathic entity. Because of its broad ing, the physical therapist is the leader, team approach.
differential diagnosis, etiology, and prog- with the physician supporting for medi-
nosis, rehabilitation must be tailored cal management of tone and spasticity
to the specific setting in which TM oc- as needed). The rehabilitation team tra-
curred. Age at onset should also be ditionally includes physicians, thera-
taken into account. This article focuses pists, nurses, case managers, and family
on idiopathic TM. members and may also include home
Long-term follow-up data on patients health aides, psychologists, respiratory
with TM reveal that approximately one- therapists, speech-language pathologists,
third of patients recover with little to and orthotists, depending on specific pa-
no sequelae, one-third are left with a tient needs and rehabilitation goals.
moderate degree of permanent disabil- Physical and occupational therapists
ity (eg, mild spasticity but independent train patients with TM to increase their
ambulation, urgency and/or consti- strength and joint range of motion
pation, some sensory deficits), and (ROM), improve tone, mitigate pain,
one-third have severe disabilities (eg, and maximize functional mobility.
inability to walk or severe gait distur- Equipment evaluation should be a reg-
bance, absence of sphincter control, ular and systematic feature of neuro-
sensory deficits).2,3 It is extremely dif- rehabilitation to determine whether
ficult to predict at onset into which any equipment changes or modifica-
category a patient will fall. Certain tions will augment and optimize func-
surrogate markers have been identified tional performance. A thorough home
to guide this prediction. For example, rehabilitation program is developed to
acute illness requiring ventilator assis- facilitate the patient’s mobility gains in
tance is re lated to later impairment in the home setting and complement the

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Copyright @ 2011 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Rehabilitation

KEY POINTS
h Some muscle tightness training done at the therapy site. The sine 5¶-triphosphate and creatine phos-
may be desirable in patient and caregiver are educated on phate; and increased levels of myofibril-
specific locations, the home program and expectations lar proteins. A strength-training regimen
such as tightness of the upon hospital discharge. may include static or dynamic training
finger flexors to produce Achieving adequate joint ROM is performed through isometric, isotonic,
strong tenodesis grasp necessary and is facilitated through and isokinetic techniques, according to
in an individual with C6 stretching exercises, use of appropriate targeted goals and the degree of neuro-
tetraplegia or tightness orthoses, and strengthening exercises. logic deficit. Training programs using
of back extensors to The most common limitations in move- the DeLorme or Oxford6 methods are
assist triceps paralysis to ment due to contractures in traumatic adapted specifically for individuals with
regain upright sitting.
and nontraumatic spinal cord disease paralysis.
h A strength-training (SCD) involve shoulder external rota- Training activities done on an adjust-
regimen may include tion, shoulder elevation, scapular de- able therapy mat are often composed of
static or dynamic pression, scapular retraction, elbow sequenced activities that progress from
training performed
extension and supination, hip exten- the easiest to the most difficult. The
through isometric,
sion, ankle dorsiflexion, and great toe usual progression is from bed mobility
isotonic, and isokinetic
techniques, according
flexion. Some muscle tightness may be to rolling, prone lying, long sitting,
to the targeted goal and desirable in specific locations, such short sitting, and sitting up from a lying
the degree of as tightness of the finger flexors to position. Muscles needed for individ-
neurologic deficit and produce strong tenodesis grasp in an uals with lower limb paralysis to be able
adapted specifically for individual with C6 tetraplegia, thereby to move or position their legs in bed are
each individual with resulting in good wrist extension func- wrist extensors, biceps, anterior deltoid,
paralysis. tion, or tightness of back extensors to middle deltoid, and shoulder girdle
assist triceps paralysis and to regain stabilizers. Individuals with tetraplegia
upright sitting. A limited amount of are taught to use their arms, head, and
overstretching can likewise help in neck for momentum to roll in bed,
specific areas, such as the increased keeping the elbow straight while the
hip external rotation required to put shoulder is flexing across the body.
on socks and shoes. Aggressive ROM Sitting up from a lying position is a
exercises are contraindicated or should prerequisite for independent dressing
be done with caution in the presence of and transfers. A thorough evaluation
unstable fractures, active heterotopic should be conducted to determine the
ossification, deep vein thrombosis, and most appropriate transfer technique for
osteoporosis. Daily stretching of mus- any individual with paralysis. Depen-
cles with terminal sustained stretch is dent transfers include sliding transfers,
considered first-line rehabilitative treat- the dependent standing pivot tech-
ment for limitation of motion due to nique, or the use of Hoyer lifts. Trans-
spasticity. fers that require some active patient
To target muscle strengthening and participation include the two-man lift,
facilitation exercises in individuals with the sliding board transfer, or the assis-
SCD, all spared or intact muscles are ted standing pivot transfer. The goal of
given progressive resistive exercises, an assisted transfer is to gradually re-
active-assistive ROM, or active ROM duce the assistance required until the
through the maximum range. Adaptive patient can perform the transfer inde-
muscle changes seen in SCD with exer- pendently. Floor-to-chair transfer train-
cise training include increased cross-sec- ing is very important for anyone who
tional area, predominantly of type II falls out of a wheelchair or otherwise
fibers; increased metabolic capacity due ends up on the floor and needs to get
to increased concentrations of adeno- back into a chair.

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Copyright @ 2011 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
KEY POINTS
Ambulation is a commonly ex- and hip stability (while the shoulder is h Individuals with an
pressed goal of most people with TM. adducted, the latissimus dorsi can act as injury at the T12 to L2
Patients with injuries at T2 and above a hip extensor, given its fixed insertion level can master
typically cannot achieve ambulation, and reverse origin, locking the hip in household ambulation,
whereas patients with T3 to T11 af- extension against its own ligaments). while individuals with L3
fectation are able to use braces for During gait training, control of the and lower injuries can
physiologic standing and therapeutic pelvis is a critical factor in successful achieve community
ambulation. The goal for individuals ambulation. Gait training includes prac- ambulation.
with a T12 to L2 injury is to achieve ticing standing activities between paral- h During a preYgait
ambulation in the household, while pa- lel bars and practicing the gait pattern training program,
tients with injuries at L3 and lower are inside and outside the bars with assis- important muscles to
most likely to achieve ambulation in tive devices (ie, canes, bilateral fore- strengthen are core
the community (distance of more than arm or axillary crutches, walkers), over trunk and abdominal
150 feet at a time). It should be noted, obstacles, on rough terrain or uneven muscles, shoulder
depressors and scapula
however, that patients with T12 to L2 ground, on ramps, on curbs, going
stabilization muscles,
injuries can also achieve community am- through doorways, and when getting
and triceps and wrist
bulation if they are young and highly into a car. Training on falling techniques extensors.
motivated. includes learning how to fall safely and
The attainment of muscle function in h During gait training,
how to get up from the floor. Finally,
control of the pelvis is a
the back and lower extremities helps prescribing the appropriate wheelchair
critical factor in
physical therapists predict the bracing for the level of the deficit, the individu- successful ambulation.
needs to aid in ambulation. For ex- al’s needs and comfort, and any medical-
ample, patients with pelvic control and orthopedic comorbidities is essential.
intact quadratus lumborum and ab-
dominal muscles may walk using long
leg braces (knee-ankle-foot orthoses ACTIVITY-BASED RESTORATIVE
[KAFOs]) and crutches. Patients with THERAPIES IN TRANSVERSE
some control of muscles crossing the MYELITIS
hip joint, such as iliopsoas, hip adduc- Activity-based restorative therapy (also
tors, sartorius, and gluteus maximus, known as activity-based therapy or
should be able to walk a limited amount activity-based rehabilitation) addresses
with long leg braces (KAFOs) and the impairment and is based on
crutches. Patients with intact quadriceps activity-dependent neural plasticity, in
can walk full-time with short leg braces which changes in the nervous and mus-
(ankle-foot orthoses [AFOs]). Patients cular system are driven by repetitive
with intact tibialis anterior and posterior activation of the neuromuscular system
can walk full-time with short leg braces above and below the injury level. The
(AFOs, supramalleolar orthoses) or no tools of activity-based restorative ther-
braces at all. apy are the same as those used in tra-
During the preYgait training pro- ditional rehabilitation, but the premise
gram, important muscles to strengthen of application is based on the nervous
are core trunk and abdominal muscles, system’s dependence on activity for
shoulder depressors and scapula sta- everything from myelination and re-
bilization muscles, and triceps and wrist myelination to new cell birth and syn-
extensors. In this phase, a structured apse formation and, ultimately, to func-
progressive resistive exercise program tion. Thus, motor activation (patterned
for the latissimus dorsi, pectoralis, lower and nonpatterned) and sensory stimu-
trapezius, and serratus anterior muscles lation as part of the rehabilitation pro-
is very important to achieve good trunk cess are done with the ultimate goal of

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Copyright @ 2011 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Rehabilitation

KEY POINTS
h Activity-based restorative neural restoration, not just function increase in cell birth in the lumbar
therapy addresses the restoration. spinal cord. In this model, FES doubled
impairment and is based the proportion of the newly born cells
on activity-dependent Motor Activation expressing markers suggestive of tripo-
neural plasticity, in Patterned motor activation can be done tential progenitors. These data suggest
which changes in the through both task-specific and nonspe- that controlled electrical activation of
nervous and muscular cific training. the CNS may enhance spontaneous re-
system are driven by Task-specific training is easy to un- generation after neurologic injuries.10
repetitive activation of derstand and has been practiced since In another study, electrical stimulation
the neuromuscular applied to the cortical pyramids in rats
the beginning of rehabilitation. Loco-
system above and below
motor training (ie, gait training) is the enhanced synapse formation in the
the injury level. By using
best example of patterned, task-specific spinal cord during development and
activity-based restorative
therapy as part of the training. It can be practiced in a partially following corticospinal tract injury.11
rehabilitation process, weight-supported environment using Elegant studies have clearly demon-
the ultimate goal is treadmill systems, automatic gait ro- strated that activation of the CNS is an
neural restoration, not bots, or water. It can also be practiced important variable influencing the cel-
just function restoration. in the traditional overground way. lular mechanisms associated with re-
h Activity-based restorative Two important principles of locomo- generation. In fact, these studies
therapy tools are used tor training when dealing with neuro- indicate that cellular regeneration is de-
to produce motor logic injuries need to be mentioned. pendent on activity. The role of activity
activation (patterned First, maximization of load bearing in may be even more important in con-
and nonpatterned) and lower extremities is essential, as the in- ditions in which normal activity is re-
sensory activation. crease in load bearing is associated with duced, as in TM. Examples of activity
h Activity plays a critical an increase in limb EMG activity, and playing a critical role in development
role in development and shared load bearing between upper and and plasticity include activity-dependent
neural plasticity, lower extremities decreases EMG activ- gene expression, modification of synap-
including influencing ity in lower limbs.7 Second, optimizing tic strength, synapse elimination, myeli-
gene expression and sensory cues, ensuring normal walk- nation and maintenance of myelination,
modifying synaptic ing speed, and optimizing kinematics and axonal growth. The widespread
strength, synapse dependence of development and plas-
(stance/swing, upper extremities pat-
elimination,
terned swing) play an essential role in ticity in the CNS on neural activity sug-
myelination,
maximizing functional recovery and gests that optimized neural activity
remyelination, and
axonal growth. minimizing compensation. might also be important for regenera-
Patterned nonspecific training is tion, given the common cellular mecha-
based on the principle of neural activa- nisms participating in both processes.12
tion of the central pattern generator This concept is further supported by
(CPG). Circuitry for the CPG is located evidence that increased neural activity
in the lumbar region of the spinal cord enhances multiple components of spon-
(L2 to L5).8 Limited input can activate taneous regeneration, while decreased
the CPG and produce interlimb CPG activity inhibits it.13
activation.8,9 Nonspecific patterned Activity itself has been associated
training is accomplished most easily by with neurotrophin production; thus, it
using functional electrical stimulation is conceivable that the elaboration of
(FES). neurotrophins after FES activation
FES is postulated to promote periph- below the injury level underlies the
eral and central nervous system repair biological basis for neural reorganiza-
following injury. Following complete tion and functional improvement.14
spinal cord transection in rats, lower Proven functional electrical stimula-
extremity FES induced an 82% to 86% tion benefits. Numerous publications

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Copyright @ 2011 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
KEY POINT
have demonstrated the practical ben- 18 weeks demonstrated that exercise h Functional electrical
efits of FES in individuals with se- may provide a greater training effect stimulation has shown
quelae of traumatic spinal cord injury on walking speed and endurance than proven benefits in
(SCI). Some of those benefits include FES, although the FES group per- individuals with
increased muscle mass, improved formed to a significantly higher level sequelae of traumatic
bone density, enhanced cardiovascu- with FES than without for the same spinal cord injury,
lar function, improved body composi- outcome measures. Therefore, the including increased
tion (ratio of muscle to fat), improved authors recommended studying the muscle mass, improved
bowel function, decreased spasticity, combined therapeutic effects of FES bone density, enhanced
improved glucose metabolism, and re- and exercise for this patient group.20 cardiovascular function,
improved body
ductions in bladder infection rate. In In a recent pilot trial, patients with MS
composition, improved
addition, electrical stimulation can be showed improvement on a broad
bowel function,
used in combination with other thera- array of functional and neurologic decreased spasticity,
pies to enhance functional recovery outcome measures including gait, improved glucose
from SCI. One example is the use of upper extremity dexterity, and quality metabolism, and
FES to facilitate gait.15 In a study of 70 of life. Furthermore, analysis of CSF reductions in bladder
patients, Solomonow and colleagues before FES and 3 months after initiat- infection rate.
demonstrated that 14 weeks of FES ing FES cycling revealed an enhanced
walking (3 h/wk) improved total cho- neural repair program (increased CSF
lesterol, low-density lipoprotein levels, transforming growth factor $3) and a
and hydroxyproline/creatinine ratios reduced inflammatory environment
and reduced spasticity in patients with within the CNS (decreased interferon-,,
paraplegia from SCI.16,17 In another IL-7, and IL-8).21
study, after 10 weeks of FES cycling (2 Functional electrical stimulation
to 3 sessions/wk), 18 subjects with SCI characteristics in transverse myelitis.
showed increased lean muscle mass, Successful implementation of FES to
improved American Spinal Injury Asso- result in muscle contractions requires
ciation motor and sensory scores, and an intact lower motor neuron. Tradi-
reductions in serum levels of IL-6, tu- tionally, the FES technology was used
mor necrosis factor ", and C-reactive to generate purposeful contractions of
protein (markers for inflammation). paralyzed muscles. Newer FES tech-
Limited clinical data are available nology, however, is based on applying
on the use of FES in demyelinating low-level electrical currents that acti-
disorders such as TM and multiple vate the ascending nerve fibers. Sig-
sclerosis (MS). A pilot trial of 12 nals enter the spinal cord and are
patients with MS who underwent FES switched either directly or via inter-
cycling (3 sessions/wk for 2 weeks) neurons to the lower motor neuron,
demonstrated improved spasticity but which causes the muscle to contract.
failed to show improvement in The use of FES is related not only to
strength and walking speed.18 Long- direct and visible muscle contraction
term FES bracing (3 to 12 months) for but also to the effect of neural activa-
footdrop has been shown to increase tion in the ascending sensory path-
strength and walking speed, suggest- ways and the effect of electrical
ing that it strengthens activation of current on the histology and biochem-
motor cortical areas and their residual ical characteristics of the muscle
descending connections in patients fibers.22,23 In TM, the lower motor neu-
with MS.19 A randomized trial of 44 ron is generally affected at the level of
subjects with MS and footdrop who the inflammation. For that reason,
received FES bracing or exercise for muscle contractions in response

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Copyright @ 2011 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Rehabilitation

KEY POINT
h Functional electrical to FES from levels at the injury site are ever published using standardized
stimulation does not usually limited or absent. This may be a functional measures. According to the
always produce visible transient phenomenon related to the study, ATM may have a bimodal distri-
activation of muscles in acute inflammation. In most patients, bution of age, with approximately 40%
transverse myelitis some of the lower motor neuron func- of patients being younger than 3 years
because of lower motor tion returns weeks to months following of age (compared to 10% reported in
neuron involvement. In the injury. During that period, it is im- previous studies) and another peak in
those cases, use of portant to try to avoid muscle atrophy incidence occurring between the ages
different current and its sequelae at that level. This may of 5 and 17 years. Most patients had
characteristics be attempted by using other electrical the greatest impairment during the
(ie, longer pulse width,
stimulation devices that utilize an un- acute illness period and reached their
bidirectional/rectangular,
usually long duration (ie, 200 6s) and functional nadir 2 days after the onset
or even direct current)
can prevent muscle
unusually shaped (bidirectional, rectan- of symptoms. Eighty-nine percent of
atrophy and its sequelae. gular) current impulse or by activating children had acute muscle weakness,
the muscle from a different spinal level were limited to bed or a wheelchair for
in addition to using traditional rehabili- mobility, or required ventilatory assis-
tation approaches (eg, bracing, taping, tance during this stage. Of the patients
splinting). who were nonambulatory, 52% were
able to walk at least 30 feet with or
Sensory Stimulation without the help of a walker, while
Sensory stimulation can be used to ac- only 39% of the patients who initially
tivate afferent pathways with the goal of needed ventilator assistance were able
providing information that can subse- to do so at the time of follow-up.
quently be used to perform a motor task In the acute phase, 82% of the pa-
with the direct effect being improved tients experienced bladder dysfunction
motor and sensory function.24,25 An ex- requiring catheterization, and 50% of
ample of this is sensory stimulation that the patients still required catheteriza-
enhances excitatory inflow from muscle tion at follow-up.
spindles to the motor neuron pools and Regarding functional outcome, most
depresses the inhibitory effect of the patients were independent in the skill
Golgi tendon organ. Focal sensory ac- areas measured by the Functional Inde-
tivation or stimulation and nonfocal pendence Measure (FIM) and WeeFIM
activation (eg, epidural stimulation or (FIM for children) systems: self-care, com-
intrathecal delivery of neurotrophin-3 munication, social cognition, transfers,
or brain-derived neurotrophic factor) and locomotion. However, 33% required
can also produce complex lower limb minimal to total assistance for ambula-
cyclinglike movements. In a double- tion or using the wheelchair for more
blind, randomized controlled clinical than 160 feet. More than half (54%) of
trial using whole-body vibration in children required minimal to total assis-
patients with MS, Schuhfried and col- tance for sphincter control, while 36%
leagues demonstrated improved pos- required assistance for mobility with
tural control and walking speed.26 transfers and 27% required assistance
for self-care needs. According to this
PEDIATRIC CONSIDERATIONS study, even though most patients
Approximately 20% of patients with recover adequate muscle strength and
acute transverse myelitis (ATM) are motor control for ambulation, significant
younger than 18 years of age.27 Pidcock impairments remain within important
and colleagues4 analyzed the largest co- functional domains such as transfers,
hort of pediatric patients with ATM self-care needs, and sphincter control.

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These limitations present an opportu- ity of the hip, hyperextension at the
nity for rehabilitation management to knee, poor or absent dorsiflexion, and
prevent secondary complications and fur- plantar flexion at the ankle. Bracing
ther improve function in the long term. and splinting have been prescribed to
In our clinical practice we have protect affected joints. During obser-
identified a subgroup of children who vation of some of these children for
presented with CSF and multilevel MRI over 2 years, recovery of motor func-
changes consistent with ATM. These tion has been poor despite aggressive
children subsequently recovered most outpatient and home rehabilitation
of the function that was acutely lost, programs including strengthening,
except for one extremity, either upper weight-bearing activities, gait training
or lower, that was left with persistent or fine motor skill remediation, joint
flaccid monoplegia (see Case 6-1). On stabilization, and bracing.29 All children
physical examination, the affected limb were given trials of FES cycle ergome-
has generally demonstrated intact sen- try (either upper or lower extremity).
sation but severely decreased motor In general, muscle contraction was not
function, rapid and severe muscle elicited, and, as sensation was intact,
atrophy, areflexia, and no muscle con- the children’s tolerance for FES was
traction with trials of electrical stimula- poor. Because of poor spontaneous re-
tion. Because of this unusual outcome, covery of neurologic function, these
electrodiagnostic studies have been children have been evaluated for
completed on all of these children. nerve, muscle, or tendon transfers in
Results of these studies have been the hope of improving function or at
consistent with severe motor neuron- least slowing the severe muscle and
opathy. We postulate that these chil- bone wasting. Three of our patients in
dren have had an immunologic or this subgroup have undergone nerve
inflammatory reaction to either the transfers. Two have received nerve
anterior horn cells or their proximal transfers with or without grafts to the
nerve roots, resulting in a lower motor biceps muscle with the goal of restor-
neuron pattern of paralysis. We antici- ing elbow flexion. One has had a nerve
pated that these children would dem- transfer to the quadriceps with the
onstrate long-term sequelae similar to goal of improving knee extension.
those of children affected with polio- Currently, the results of these proce-
myelitis or brachial plexus injury, dures are unknown. Referral for nerve
including asymmetric limb growth, transfers must be made early in the
weakness, joint dislocation or contrac- course of recovery, as experience with
ture, scoliosis, and scapular winging or the neonatal and traumatic nerve
gait abnormalities.28 Upper extremity injury populations shows that better
fractures (multiple in several children), outcomes are achieved if these proce-
including buckle fractures in the wrists, dures are performed within 3 to 9
have been observed in several children months, as fewer muscle fibers and
with plegic upper extremities due to less motor endplate atrophy will be
rapid onset and progression of disuse present.30 The goal of muscle and ten-
or neurogenic osteoporosis. Indeed, don transfer procedures is to stabilize
children with affected upper extrem- joints (such as the shoulder or ankle)
ities demonstrated shoulder subluxa- and to potentially improve function
tion, scapular winging, and mild (such as elbow flexion or ankle plantar
scoliosis, and children with lower ex- flexion or dorsiflexion). Because these
tremity involvement demonstrated lax- procedures are not as time-sensitive,

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Rehabilitation

Case 6-1
A 6-year, 8-month-old boy (Patient 1) presented
with sudden-onset right upper and lower limb
weakness with associated pain and muscle
spasm. A 3-year, 9-month-old girl (Patient 2)
presented with left upper limb weakness
progressing to left lower limb weakness,
pain, and spasms. Acutely, both patients
demonstrated gait disturbance, and neither
lost bowel or bladder function. Both children
reported symptoms of a mild viral illness several
days prior to the onset of paresis. Neither child
had a personal or family history of neurologic
or autoimmune disorders. In both cases, CSF
analysis was consistent with inflammation
(Patient 1 had a white blood cell count of 138, a FIGURE 6-1 T2-weighted axial and sagittal MRI images of
protein of 35 mg/dL, and a glucose of 60 mg/dL; the cervical spine depicting Patient 1 (A) and
Patient 2 (B). A hyperintense T2 signal is present within the
Patient 2 had a white blood cell count of 21, a cervical spinal cord ranging from level C2 through T1 in
protein of 26 mg/dL, and a glucose of 51 mg/dL). Patient 1 and C3 through C7 in Patient 2 (white arrows),
Workup for other causes of myelopathy, corresponding with cervical gray matter areas.
including viral and bacterial cultures, oligoclonal
bands, and serum neuromyelitis optica titers was negative. In both cases, MRI revealed multilevel
cervical T2 changes consistent with spinal cord edema (Figure 6-1). Ultimately, a diagnosis of transverse
myelitis was made in both cases. Patient 1 was treated with antibiotics, antiviral medication, and IV
immunoglobulin. Patient 2 was treated with IV methylprednisolone sodium succinate for 5 days with
taper over 6 weeks. Both recovered full lower extremity motor function within 10 days; however,
unilateral upper extremity paresis persisted.
Subsequent electrodiagnostic studies revealed
evidence of severe motor neuronopathy.
Evaluation of the affected upper extremity 14
months (Patient 1) and 3 months (Patient 2)
after onset demonstrated areflexia at the biceps,
triceps, and brachioradialis muscles with flaccid
tone and severe wasting of proximal and distal
shoulder muscles (Figure 6-2 and Table 6-1).
Sensation was intact and no residual pain was
reported. Significant shoulder subluxation in the
affected limb and upper limb residual paresis
were present in the proximal and distal muscle
FIGURE 6-2 Photographs of Patient 1 (A) and Patient 2 (B)
depicting muscular wasting and disuse
groups with sparing of the finger flexors. Both
atrophy when compared to the unaffected extremity.
children underwent 2 weeks of intensive Glenohumeral subluxation is notable, as is shoulder girdle and
occupational therapy, which included functional trunk asymmetry. Scoliosis is evident in both cases, indicating
electrical stimulation (FES)-assisted upper that the posterior rami are affected.
extremity (UE) ergometry, scapular stabilization
exercises, supported UE weight bearing, and fine motor skill remediation. Mechanical approximation of
the glenohumeral joint was also included, either via FES or supportive bracing. FES-assisted UE
ergometry was performed on the RT300-SA arm cycle, and the deltoids, rotator cuff, biceps, triceps,
finger flexors, and extensors were stimulated sequentially. The children’s tolerance for FES was poor, as
sensation was intact. Alternate exercises were performed with conventional facilitation techniques. A
home therapy program was developed consisting of closed-chain proximal strengthening, fine motor
Continued on page 825

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Continued from page 824
skill remediation,
and mechanical TABLE 6-1 Evaluation Prior to Onset of Rehabilitation
approximation of
the glenohumeral Patient 1 Patient 2
joint, either via FES Physical Examination
or supportive Reflexes Absent Absent
bracing. Both
children continued Muscle bulk Decreased Decreased
with land and Muscle tone Flaccid Flaccid
aquatic therapy at
home twice a week. Pain None None
Patient 1 continued Sensation Intact Intact
with FES-assisted UE
Bowel and bladder Continent Continent
cycling twice a week.
Long-term follow-up Strength (Manual muscle test 0 to 5, 5 indicating full strength)
(20 months for Shoulder flexion 1 0
Patient 1 and 18
Shoulder extension 1 0
months for Patient 2)
demonstrated Shoulder abduction 1 0
persistence of some Shoulder adduction 1 0
UE motor dysfunction
in both patients. Both Shoulder external rotation 1 1
had near-normal Shoulder internal rotation 1 3
finger and wrist
flexion with moderate Elbow flexion 3 0
wrist extension Elbow extension 0 0
strength and had
Forearm supination 1 0
recovered 50% of
active antigravity Forearm pronation 1 0
internal and external Wrist flexion 1 3
rotation at the
shoulder. Patient 1 Wrist extension 1 1
was able to produce Finger DIP extension 2 1
50% of active
Finger DIP flexion 2 4
antigravity shoulder
abduction strength, Finger abduction 0 0
while Patient 2 Finger adduction 0 0
demonstrated 30%
improvement in Opposition 0 1
motor strength
DIP = distal interphalangeal.
output. Because of
slow motor recovery, both children were evaluated for nerve or tendon transfers. Patient 1 was deemed a
candidate for tendon transfers, but the family decided against it. Patient 2 underwent nerve transfers including
spinal accessory nerve to suprascapular nerve and median nerve fascicle to biceps nerve.
Comment. Persistent isolated unilateral UE paresis is a unique complication of transverse myelitis
and is most likely due to injury to anterior horn cells. Restoration of motor function is limited
despite aggressive rehabilitation, and rehabilitation is complicated by intact sensory function. Early
consideration for surgical reconstruction (ie, nerve or tendon transfers) is appropriate.

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Rehabilitation

they may be delayed to allow for addi- spasticity, deep vein thrombosis, pulmo-
tional spontaneous recovery. nary embolisms,35 and, in postpubertal
As in adults, most children with ATM adolescents, neurogenic erectile dysfunc-
will require rehabilitative measures to tion.36 If not properly managed, venous
maximize recovery and manage sec- thromboembolic events are a common
ondary complications from the resul- cause of morbidity and mortality follow-
tant SCI, paralysis, and immobility. ing any SCI.35 With high injuries, ability
Occupational and physical therapy to control the diaphragm is lost, and
should be started as soon as medi- long-term mechanical ventilator assis-
cally possible to prevent contractures, tance for respiratory management is
muscle atrophy, and skin breakdown. required. For patients with cervical or
During the acute stage, therapeutic higher thoracic lesions (above T6), au-
modalities should include stretching, tonomic dysreflexia is a life-threatening
strengthening, and sitting and standing potential medical complication.37
balance activities. Skilled inpatient and In addition, patients develop bladder
outpatient physical and occupational and bowel incontinence, commonly
therapy helps to strengthen weak mus- referred to as neurogenic bladder and
cles, improve ROM, and minimize de- bowel. Incontinence can be a source of
conditioning. In the inpatient setting, embarrassment for older children and
the emphasis is on maximizing inde- adolescents, and its successful man-
pendence with activities of daily living agement necessitates considerable
(ADLs), transfers, and mobility with the adherence on their part. To prevent
provision and use of appropriate med- secondary complications from a neuro-
ical equipment (such as wheelchairs, genic bladder, such as low bladder
transfer boards, bathroom equipment, compliance, upper tract changes, and
adaptive equipment, etc.). The patient renal deterioration, children should
should be fitted for braces (resting hand have renal function monitoring with a
splints and AFOs) to maintain passive renal ultrasound within the first 3
ROM, if necessary. In the outpatient months of onset, urodynamic testing
setting, the emphasis is on fine motor within the first 6 months, and early
skills, proper transfer techniques, and, institution of a clean intermittent cath-
if possible, advanced gait training. De- eterization program.38 Monitoring is of
velopmental milestones should be in- special importance because neurologic
corporated into the patient’s functional examination or urinary symptoms can-
and rehabilitative goals. Mobility is a not accurately predict renal deteriora-
major issue that is addressed initially tion. This is important to remember
and periodically as the patient’s con- because the ability to ambulate or the
dition changes. presence of lower extremity tone does
The loss of sensory and motor con- not correlate with urodynamic find-
trol after ATM contributes to a range of ings. The patient’s ability to void vol-
secondary medical complications that untarily does not mean that bladder
must be prevented or managed to max- function is normal.38
imize the recovery potential. Special at- Muscle spasticity can be a very dif-
tention is required for prevention of ficult problem to manage. The primary
pressure ulcers31 (with adequate posi- goal is to maintain adequate passive and
tioning and pressure releases), nutrition- active ROM. This is usually achieved by
al inadequacy, metabolic complications a daily stretching program and incorpo-
(such as early-onset hypercholesterole- ration of necessary bracing. Splints can
mia32,33 and obesity34), osteoporosis, be fabricated for ankles, wrists, elbows,

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KEY POINT
and knees. Sometimes pharmacologic to take full advantage of adequate h In young children with
intervention is required. Oral antispas- seating systems, spinal orthoses, and transverse myelitis,
ticity medications can be used for a functional strengthening program to skeletal abnormalities
generalized spasticity. For patients with improve trunk support and sitting (ie, scoliosis, hip
local spasticity, chemodenervation pro- balance. dysplasia) are expected
cedures, such as botulinum toxin injec- Bony torsional deformities usually and should be
tions or phenol blocks, may be of use. develop because of muscle weakness aggressively monitored
Some children have very difficult-to- and abnormal tone. Although experts and corrected.
manage spasticity and tone, and addi- believe that no amount of splinting,
tional interventions, such as intrathecal stretching, torsional cables, or exercise
baclofen pump implantation, may need may reverse these deformities, some
to be considered. The ultimate goal of limited studies have demonstrated that
these medicorehabilitative interven- timely use of botulinum toxin injections
tions is to assist function by relaxing combined with a comprehensive reha-
overpowering spastic muscles and bilitative program may help prevent or
strengthening weaker muscles, thereby halt the progression in children with
facilitating specific ADLs. It is essential neuromuscular disorders.42
to encourage additional activities, espe- Muscle imbalance, spasticity, con-
cially aerobic conditioning and an over- tractures, and limited ambulation may
all strengthening program, to aid also lead to inadequate acetabular for-
development of new skills and recovery mation, femoral head subluxation, and
of the CNS.39 dislocation. Problems with hip sublux-
As mentioned, younger age of on- ation or dislocation may occur in up to
set, specifically under the age of 3 82% of children with paralysis from TM,
years, has been associated with a depending on the age of onset (more
worse outcome.4 In addition to im- common in younger children) and the
pairments in ADLs and bowel and severity of disabilities. Hip ROM, limb
bladder control concerns, developmen- length discrepancies, or ‘‘popping’’
tal implications should be addressed sounds need to be assessed during
during the rehabilitative phase in this regular examination, as these may
patient population. Adequate position- result in difficulty with seating, posi-
ing and support are essential because tioning, transfers, perineal hygiene,
muscle weakness, spasticity, and lim- dressing, pressure ulcers, or pain. Ther-
ited weight bearing can lead to mus- apeutic conservative measures should
culoskeletal complications such as include physical therapy and daily
scoliosis, rotational (torsional) deform- stretching exercises, positioning, weight-
ities and hip dysplasia, subluxation, or bearing activities, and use of orthotic
dislocation. Nearly all children with devices. A variety of orthotic devices
ATM (especially children younger than that attempt to keep the patient’s hip
5 years of age) will develop scoliosis in an abducted and neutral-to-external-
if disease onset occurs prior to the rotation position are available. This
adolescent growth spurt.40 This can abduction bracing is contraindicated in
lead to pelvic obliquity, problems with patients who have lower motor neuron
sitting balance, skin breakdown, and findings, flaccid paralysis, or fixed
pain. If scoliosis progresses and be- deformities. Standing and walking activ-
comes more severe it may impair ities should be encouraged because
respiratory function because of de- these have been shown to delay or
creased lung volumes and inefficient prevent hip subluxation or dislocation in
diaphragmatic exertion.41It is essential children.43

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Rehabilitation

KEY POINT
h Applying conceptually It is essential for the family to be chronic condition that needs ongoing,
sound principles of educated in the impairments that the targeted, and well-thought-out inter-
neural restoration patient may experience and in the ventions in order to optimize outcomes
through rehabilitation provision of a home exercise or therapy and improve function and quality of life.
and using rehabilitation program that they can follow to main- Studying, applying, and ‘‘dosing’’ exer-
as essential treatment, tain good health and continue to pro- cise and rehabilitative interventions as a
rather than as an mote recovery through the years. means to repair damage along a treat-
afterthought to the Because community reintegration, ad- ment paradigm only makes sense. The
neurologic injury, could vanced education, and functional inde- treatment paradigm, however, is not
allow for greater pendence have been associated with static, but is continuously evolving, es-
functional gains and
higher life satisfaction and better qual- pecially with the addition of immune-
better neural repair.
ity of life,44,45 proactive psychological modulatory therapy. Because of the
Exercise and
rehabilitation can be
interventions can focus on increasing chronic nature of paralysis and its sig-
used as interventions to participation levels. While increasing nificant medical and surgical comorbid-
facilitate neurorestoration functional independence, mobility, so- ities, integrating activity into the daily
by applying sound cial interaction, and community skills home life of individuals affected by SCD
neurophysiologic is important, it is also important to is the only pragmatic approach. Edu-
principles rooted in discuss adulthood transition issues cation and ongoing reevaluation and
neural plasticity, cell early in the care of patients with SCI therapeutic changes provided in a medi-
birth, myelination/ from TM. Patients and families should cal center are complemented by home-
remyelination, and be provided with the necessary tools based rehabilitative programs designed
synapse formation, all of for decision making, including com- to be functional and feasible in the con-
them activity dependent.
munity integration and participation, text of daily living and to scientifically
in every developmental stage. Activities target injured structures. The goal of
that can facilitate skill development rehabilitation is to optimize neurologic
and integration include specialized recovery while waiting for a cure for the
camps, adaptive sports, school activ- damaged spinal cord.
ities, sleepovers, and exercise pro-
grams, among others.46 USEFUL WEBSITES
International Center for Spinal Cord
CONCLUSION Injuries at Kennedy Krieger Institute
This article presented an update con- www.spinalcordrecovery.org.
cerning rehabilitative interventions in Christopher & Dana Reeve Foundation
TM to bring the ‘‘bench’’ concepts to NeuroRecovery Network
the bedside. The nervous system is dy- www.christopherreeve.org/site/c.
namic and continually changing, and ddJFKRNoFiG/b.5399929/k.6F37/
maintaining optimal activity is essential NeuroRecovery_Network.htm.
for neural regeneration and restoration.
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